First report of macrophage activation syndrome in hyperimmunoglobulinemia D with periodic fever syndrome

Donato Rigante, Antonio Chiaretti, Marco Piastra, Silvia Maria Pulitano', Ettore Domenico Capoluongo, Valentina Ansuini, Orazio Genovese, Achille Stabile, Barbara Bertoni, Adele Compagnone

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49 Citazioni (Scopus)

Abstract

We describe for the first time a case of macrophage activation syndrome in a child with hyperimmunoglobulinemia D with periodic fever syndrome who required intensive care support. Up-regulated monokine production, high serum levels of triglycerides and ferritin, clotting abnormalities with hypofibrinogenemia, and rapidly evolving pancytopenia should alert the clinician to the possible diagnosis of macrophage activation syndrome, even in autoinflammatory diseases characterized basically by the periodic recurrence of unprovoked inflammatory attacks. Bone marrow aspiration showing well-differentiated macrophages phagocytosing hematopoietic elements remains the main tool for a final diagnosis, and cyclosporine is the best strategy for treatment.
Lingua originaleEnglish
pagine (da-a)658-661
Numero di pagine4
RivistaArthritis and Rheumatism
Volume2007
Stato di pubblicazionePubblicato - 2007

Keywords

  • hyperIgD-syndrome
  • macrophage activation syndrome

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